Agenesis of the right lobe of the liver is an extremely rare congenita
l anomaly, usually accompanied by additional anomalies such as a retro
hepatically or suprahepatically located gallbladder. In this report we
present a new case of agenesis of the right lobe of the liver with th
e Chiliaditi syndrome and a subdiaphragmatic hydatid cyst, diagnosed b
y CT and CT-cholangiography.